Perioral myoclonia with absences (POMA) was first described in 1994 by CP Panayotopoulos who identified 6 cases that did not fit with the classical syndrome of absence epilepsy in children and whose ... [more ▼]

Perioral myoclonia with absences (POMA) was first described in 1994 by CP Panayotopoulos who identified 6 cases that did not fit with the classical syndrome of absence epilepsy in children and whose predominant symptom during the absence seizure was the occurrence of myoclonia of perioral muscles. The POMA belongs to the group of generalized idiopathic epilepsies. It begins in childhood and there is a female predominance. It may be accompanied by tonic-clonic generalized seizures as well as absence status epilepticus. It has the EEG characteristics of typical absence seizures and therefore remains currently considered as such. The clinical manifestations of POMA are often misdiagnosed as focal motor seizures. This syndrome can be pharmacoresistant and is not likely to regress spontaneously. We present two clinical observations of perioral myoclonia with absences. The first case illustrates the typical electro-clinical features of this syndrome while the second illustrates its pharmacoresistance. [less ▲]

In Sub-Saharan Africa, intrarectal diazepam is the first-line anticonvulsant mostly used in children. We aimed to assess this standard care against sublingual lorazepam, a medication potentially as ... [more ▼]

In Sub-Saharan Africa, intrarectal diazepam is the first-line anticonvulsant mostly used in children. We aimed to assess this standard care against sublingual lorazepam, a medication potentially as effective and safe, but easier to administer. A randomized controlled trial was conducted in the pediatric emergency departments of 9 hospitals. A total of 436 children aged 5 months to 10 years with convulsions persisting for more than 5 minutes were assigned to receive intrarectal diazepam (0.5 mg/kg, n = 202) or sublingual lorazepam (0.1 mg/kg, n = 234). Sublingual lorazepam stopped seizures within 10 minutes of administration in 56% of children compared with intrarectal diazepam in 79% (P < .001). The probability of treatment failure is higher in case of sublingual lorazepam use (OR = 2.95, 95% CI = 1.91-4.55). Sublingual lorazepam is less efficacious in stopping pediatric seizures than intrarectal diazepam, and intrarectal diazepam should thus be preferred as a first-line medication in this setting. [less ▲]

Aim We report three cases of Landau-Kleffner syndrome (LKS) in children (two females, one male) in whom diagnosis was delayed because the sleep electroencephalography (EEG) was initially normal. Method ... [more ▼]

Aim We report three cases of Landau-Kleffner syndrome (LKS) in children (two females, one male) in whom diagnosis was delayed because the sleep electroencephalography (EEG) was initially normal. Method Case histories including EEG, positron emission tomography findings, and long-term outcome were reviewed. Results Auditory agnosia occurred between the age of 2 years and 3 years 6 months, after a period of normal language development. Initial awake and sleep EEG, recorded weeks to months after the onset of language regression, during a nap period in two cases and during a full night of sleep in the third case, was normal. Repeat EEG between 2 months and 2 years later showed epileptiform discharges during wakefulness and strongly activated by sleep, with a pattern of continuous spike-waves during slow-wave sleep in two patients. Patients were diagnosed with LKS and treated with various antiepileptic regimens, including corticosteroids. One patient in whom EEG became normal on hydrocortisone is making significant recovery. The other two patients did not exhibit a sustained response to treatment and remained severely impaired. Interpretation Sleep EEG may be normal in the early phase of acquired auditory agnosia. EEG should be repeated frequently in individuals in whom a firm clinical diagnosis is made to facilitate early treatment. [less ▲]

Summary : Febrile Seizures (FS), despite their usual benign clinical course, are still subject of controversies regarding the need for further investigation and treatment with anti-epileptic drugs (AEDs). Our study aimed to inventory the clinical findings, laboratory and imaging data associated with FS and eventually influencing their management. 275 episodes admitted with FS at the emergency ward of the Liège CHR over a 5 year period were retrospectively analyzed regarding precipitating factors; clinical features; laboratory, electroencephalographic, and imaging studies; as well as treatment response. FS represented 1.4% of admissions to the pediatric service. 31.3% of patients had a family history of seizure disorder. 9% percent of seizures were focal, 11.7% recurrent, and 12.3% prolonged (greater than 10 minutes). Upper respiratory tract and otorhinolaryngologic viral infections were the most often implicated provoking factors, occurring in 69.5% of patients. Laboratory, electroencephalographic and radiographic studies were normal in more than 90% of cases. 73.8% of seizures resolved without intervention. An AED was required to manage the remaining 26.2%. This study confirms the favorable outcomes of FS as demonstrated in previous studies. This happens without requiring AEDs for resolution, and without recurrence. Laboratory, electroencephalographic and imaging studies, as well as initiation of AEDs should be based primarily on clinical severity. [less ▲]

Summary : Down syndrome is one of best caracterized genetic syndromes. Its phenotype includes specific morphological features, mental retardation and several organic impairments such as cardiac ... [more ▼]

Summary : Down syndrome is one of best caracterized genetic syndromes. Its phenotype includes specific morphological features, mental retardation and several organic impairments such as cardiac malformations, cutaneous disorders and immunologic abnormalities. Beside these disturbances neurological disorders have been less considered or thought to be the expression of the mental retardation. However it is important to pay attention to the high incidence of epilepsy such as West syndrome in infants and “Late-Onset of myoclonic epilepsy in Down Syndrome” in the adult. They might required specific treatment. ADHD as well as autistic behavior may be difficult to diagnose especially in children with severe mental retardation. They need precise attention and rigourous follow-up of their treatment. [less ▲]

Prematurity remains a public health problem with a considerable psychosocial impact. Premature infants are discharged home more fragile and more precociously than infants born at term. Post-discharge ... [more ▼]

Prematurity remains a public health problem with a considerable psychosocial impact. Premature infants are discharged home more fragile and more precociously than infants born at term. Post-discharge nutrition and growth of the preterm infants should be carefully followed because of specific needs of these infants. Infections and cardiorespiratory abnormalities are more frequent in ex-premature infants. Some cerebral lesions may be shown by brain imaging suggesting future sequelae. However, estimation of their real consequences remains imperfect and long term prognosis contains many uncertainties. Cerebral palsy seems to be less severe nowadays, but all current gravity is due to disabilities which express later: hearing disorders, visual impairments, alterations of eye-hand coordination skills, attention deficit disorders, psychological troubles and school difficulties. Multidisciplinary consultations are designed for these children because early screening and adapted care can improve long term prognosis. All this underlines the importance of prolonged follow-up program after discharge for premature infants and others who presented worse suffer from hypoxic/ischemic encephalopathy. [less ▲]

The most recent antiepileptic drugs used in children are lamotrigine, topiramate, oxcarbamaz6pine and levetiracetam. Their efficacy is proven, depending on the type of crisis, but in Belgium they are ... [more ▼]

The most recent antiepileptic drugs used in children are lamotrigine, topiramate, oxcarbamaz6pine and levetiracetam. Their efficacy is proven, depending on the type of crisis, but in Belgium they are reimbursed only in certain conditions. The treatment of children with attention deficit hyperactivity disorder (ADHD), which was only constituted of methylphenidate, can now benefit from atomoxetine whose mechanism of action is different. [less ▲]

The authors propose to define the epileptic syndromes with continuous spikes and waves during slow sleep (CSWS) as a cognitive or behavioral impairment acquired during childhood, associated with a strong ... [more ▼]

The authors propose to define the epileptic syndromes with continuous spikes and waves during slow sleep (CSWS) as a cognitive or behavioral impairment acquired during childhood, associated with a strong activation of the interictal epileptiform discharges during NREM sleep--whatever focal or generalized--and not related to another factor than the presence of CSWS. The type of syndrome will be defined according to the neurological and neuropsychological deficit. These syndromes have to be classified among the localization-related epileptic syndromes. Some cases are idiopathic and others are symptomatic. Guidelines for work-up and treatment are proposed. [less ▲]